403 related articles for article (PubMed ID: 20147319)
1. Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Custer SK; Neumann M; Lu H; Wright AC; Taylor JP
Hum Mol Genet; 2010 May; 19(9):1741-55. PubMed ID: 20147319
[TBL] [Abstract][Full Text] [Related]
2. Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.
Weihl CC; Dalal S; Pestronk A; Hanson PI
Hum Mol Genet; 2006 Jan; 15(2):189-99. PubMed ID: 16321991
[TBL] [Abstract][Full Text] [Related]
3. TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
Ritson GP; Custer SK; Freibaum BD; Guinto JB; Geffel D; Moore J; Tang W; Winton MJ; Neumann M; Trojanowski JQ; Lee VM; Forman MS; Taylor JP
J Neurosci; 2010 Jun; 30(22):7729-39. PubMed ID: 20519548
[TBL] [Abstract][Full Text] [Related]
4. The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.
Nalbandian A; Donkervoort S; Dec E; Badadani M; Katheria V; Rana P; Nguyen C; Mukherjee J; Caiozzo V; Martin B; Watts GD; Vesa J; Smith C; Kimonis VE
J Mol Neurosci; 2011 Nov; 45(3):522-31. PubMed ID: 21892620
[TBL] [Abstract][Full Text] [Related]
5. Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.
Weihl CC; Miller SE; Hanson PI; Pestronk A
Hum Mol Genet; 2007 Apr; 16(8):919-28. PubMed ID: 17329348
[TBL] [Abstract][Full Text] [Related]
6. Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
Chang YC; Hung WT; Chang YC; Chang HC; Wu CL; Chiang AS; Jackson GR; Sang TK
PLoS Genet; 2011 Feb; 7(2):e1001288. PubMed ID: 21304887
[TBL] [Abstract][Full Text] [Related]
7. The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology.
Nalbandian A; Llewellyn KJ; Kitazawa M; Yin HZ; Badadani M; Khanlou N; Edwards R; Nguyen C; Mukherjee J; Mozaffar T; Watts G; Weiss J; Kimonis VE
PLoS One; 2012; 7(9):e46308. PubMed ID: 23029473
[TBL] [Abstract][Full Text] [Related]
8. Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
Ju JS; Fuentealba RA; Miller SE; Jackson E; Piwnica-Worms D; Baloh RH; Weihl CC
J Cell Biol; 2009 Dec; 187(6):875-88. PubMed ID: 20008565
[TBL] [Abstract][Full Text] [Related]
9. Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.
Ju JS; Weihl CC
Hum Mol Genet; 2010 Apr; 19(R1):R38-45. PubMed ID: 20410287
[TBL] [Abstract][Full Text] [Related]
10. Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms.
Chan HT; Lee TR; Huang SH; Lee HY; Sang TK; Chan HL; Lyu PC
Mol Biosyst; 2012 Jun; 8(6):1730-41. PubMed ID: 22481368
[TBL] [Abstract][Full Text] [Related]
11. Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
Guinto JB; Ritson GP; Taylor JP; Forman MS
Acta Neuropathol; 2007 Jul; 114(1):55-61. PubMed ID: 17457594
[TBL] [Abstract][Full Text] [Related]
12. Phenotypic variability in three families with valosin-containing protein mutation.
Spina S; Van Laar AD; Murrell JR; Hamilton RL; Kofler JK; Epperson F; Farlow MR; Lopez OL; Quinlan J; DeKosky ST; Ghetti B
Eur J Neurol; 2013 Feb; 20(2):251-8. PubMed ID: 22900631
[TBL] [Abstract][Full Text] [Related]
13. Pathological consequences of VCP mutations on human striated muscle.
Hübbers CU; Clemen CS; Kesper K; Böddrich A; Hofmann A; Kämäräinen O; Tolksdorf K; Stumpf M; Reichelt J; Roth U; Krause S; Watts G; Kimonis V; Wattjes MP; Reimann J; Thal DR; Biermann K; Evert BO; Lochmüller H; Wanker EE; Schoser BG; Noegel AA; Schröder R
Brain; 2007 Feb; 130(Pt 2):381-93. PubMed ID: 16984901
[TBL] [Abstract][Full Text] [Related]
14. Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
Rodriguez-Ortiz CJ; Hoshino H; Cheng D; Liu-Yescevitz L; Blurton-Jones M; Wolozin B; LaFerla FM; Kitazawa M
Am J Pathol; 2013 Aug; 183(2):504-15. PubMed ID: 23747512
[TBL] [Abstract][Full Text] [Related]
15. The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?
Clemen CS; Winter L; Strucksberg KH; Berwanger C; Türk M; Kornblum C; Florin A; Aguilar-Pimentel JA; Amarie OV; Becker L; Garrett L; Hans W; Moreth K; Neff F; Pingen L; Rathkolb B; Rácz I; Rozman J; Treise I; Fuchs H; Gailus-Durner V; de Angelis MH; Vorgerd M; Eichinger L; Schröder R
Biochem Biophys Res Commun; 2018 Sep; 503(4):2770-2777. PubMed ID: 30100055
[TBL] [Abstract][Full Text] [Related]
16. A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
Nalbandian A; Llewellyn KJ; Badadani M; Yin HZ; Nguyen C; Katheria V; Watts G; Mukherjee J; Vesa J; Caiozzo V; Mozaffar T; Weiss JH; Kimonis VE
Muscle Nerve; 2013 Feb; 47(2):260-70. PubMed ID: 23169451
[TBL] [Abstract][Full Text] [Related]
17. IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation.
Bayraktar O; Oral O; Kocaturk NM; Akkoc Y; Eberhart K; Kosar A; Gozuacik D
PLoS One; 2016; 11(10):e0164864. PubMed ID: 27768726
[TBL] [Abstract][Full Text] [Related]
18. Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.
Weihl CC; Pestronk A; Kimonis VE
Neuromuscul Disord; 2009 May; 19(5):308-15. PubMed ID: 19380227
[TBL] [Abstract][Full Text] [Related]
19. A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.
Erzurumlu Y; Kose FA; Gozen O; Gozuacik D; Toth EA; Ballar P
Int J Biochem Cell Biol; 2013 Apr; 45(4):773-82. PubMed ID: 23333620
[TBL] [Abstract][Full Text] [Related]
20. VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD.
Tresse E; Salomons FA; Vesa J; Bott LC; Kimonis V; Yao TP; Dantuma NP; Taylor JP
Autophagy; 2010 Feb; 6(2):217-27. PubMed ID: 20104022
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
